Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association

J Rychik, AM Atz, DS Celermajer, BJ Deal… - Circulation, 2019 - Am Heart Assoc
It has been 50 years since Francis Fontan pioneered the operation that today bears his
name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a …

Fontan-associated liver disease: screening, management, and transplant considerations

J Emamaullee, AN Zaidi, T Schiano, J Kahn… - Circulation, 2020 - Am Heart Assoc
Surgical innovation and multidisciplinary management have allowed children born with
univentricular physiology congenital heart disease to survive into adulthood. An estimated …

Intestine farnesoid X receptor agonist and the gut microbiota activate G‐protein bile acid receptor‐1 signaling to improve metabolism

P Pathak, C Xie, RG Nichols, JM Ferrell… - …, 2018 - Wiley Online Library
Bile acids activate farnesoid X receptor (FXR) and G protein–coupled bile acid receptor‐1
(aka Takeda G protein–coupled receptor‐5 [TGR5]) to regulate bile acid metabolism and …

[HTML][HTML] Fontan-associated liver disease: A review

TT Gordon-Walker, K Bove, G Veldtman - Journal of cardiology, 2019 - Elsevier
The Fontan procedure has led to increased long-term survival of patients with single
ventricle congenital heart disease. Hemodynamic changes associated with the Fontan …

The clinical spectrum of Fontan-associated liver disease: results from a prospective multimodality screening cohort

ID Munsterman, AL Duijnhouwer… - European heart …, 2019 - academic.oup.com
Aims Liver fibrosis and cirrhosis are a consequence of a Fontan physiology, and determine
prognosis. It is unclear whether non-invasive assessment of liver pathology is helpful to …

Fontan‐associated liver disease

MB Hilscher, ML Wells, SK Venkatesh, F Cetta… - …, 2022 - Wiley Online Library
Fontan-associated liver disease (FALD) is a form of congestive hepatopathy that is universal
among patients who have undergone Fontan palliation for functional single ventricle …

Fontan-associated liver disease: a review

L Téllez, E Rodríguez-Santiago, A Albillos - Annals of hepatology, 2018 - medigraphic.com
Fontan-associated liver disease is a hepatic disorder arising from hemodynamic changes
and systemic venous congestion following Fontan surgery. The histological changes …

Outcomes in hypoplastic left heart syndrome

MK Metcalf, J Rychik - Pediatric Clinics, 2020 - pediatric.theclinics.com
Congenital heart defects occur in 8 per 1000 live births, with anomalies ranging from minor
septal defects to major structural abnormalities of the great vessels or ventricles. Why focus …

Single-cell multiomics guided mechanistic understanding of Fontan-associated liver disease

P Hu, J Rychik, J Zhao, H Bai, A Bauer, W Yu… - Science translational …, 2024 - science.org
The Fontan operation is the current standard of care for single-ventricle congenital heart
disease. Individuals with a Fontan circulation (FC) exhibit central venous hypertension and …

[PDF][PDF] Assessment of advanced liver fibrosis and the risk for hepatic decompensation in patients with congestive hepatopathy

A Lemmer, LB VanWagner, D Ganger - Hepatology, 2018 - Wiley Online Library
Congestive hepatopathy (CH) arises from chronically elevated right‐sided heart pressures
transmitted to the liver by passive venous congestion. Over time, CH can lead to hepatic …